Ciwon Chediak-Higashi

Cutar Chediak-Higashi cuta ce mai saurin gaske ta tsarin rigakafi da na juyayi. Ya haɗa da gashi, idanu, da fata masu launi.

Cutar Chediak-Higashi ta wuce ta cikin iyalai (waɗanda aka gada). Yana da wani autosomal recessive cuta. Wannan yana nufin cewa iyayen biyu suna ɗaukar jigilar kwayar halittar da ba ta aiki. Kowane iyaye dole ne su ba da jinyar da ba ta aiki ba ga yaron don su nuna alamun cutar.

An sami lahani a cikin LYST (kuma ana kiranta CHS1) kwayar halitta Babban lahani a cikin wannan cutar ana samun sa ne a cikin wasu abubuwa da aka saba gabatarwa a cikin ƙwayoyin fata da wasu ƙwayoyin farin jini.

Yaran da ke wannan yanayin na iya samun:

• Gashin azurfa, idanu masu launuka masu haske (albiniya)
• Infectionsara yawan kamuwa da cuta a cikin huhu, fata, da ƙwayoyin mucous
• Movementsungiyoyin ido na Jerky (nystagmus)

Kamuwa da cutar yara da ke fama da wasu ƙwayoyin cuta, kamar su kwayar cutar Epstein-Barr (EBV), na iya haifar da rashin lafiya mai barazanar rai kamar ta kwayar cutar kansar jini.

Sauran cututtuka na iya haɗawa da:

• Rage gani
• Rashin hankali
• Raunin jijiyoyi
• Matsalar jijiya a gabobin jiki (neuropathy na gefe)
• Hancin Hanci ko rauni mai sauƙi
• Numfashi
• Tsoro
• Kamawa
• Haskakawa zuwa haske mai haske (photophobia)
• Rashin tafiya (ataxia)

Mai ba da sabis na kiwon lafiya zai yi gwajin jiki. Wannan na iya nuna alamun kumburin ciki ko hanta ko jaundice.

Gwajin da za a iya yi sun hada da:

• Cikakken ƙidayar jini, gami da ƙididdigar ƙwayar ƙwayar jini
• Countididdigar jinin jini
• Al'adun jini da shafawa
• Brain MRI ko CT
• EEG
• EMG
• Gwajin gwajin jijiyoyi

Babu takamaiman magani don cutar Chediak-Higashi. Planarin sassan ƙashi da aka yi a farkon cutar ya bayyana sun yi nasara ga marasa lafiya da yawa.

Ana amfani da maganin rigakafi don magance cututtuka. Yawancin lokuta ana amfani da magungunan ƙwayoyin cuta, kamar acyclovir, da magungunan ƙwayoyin cuta a cikin saurin cutar. Ana ba da jini da ƙarin jini yayin da ake bukata. Ana iya buƙatar yin aikin tiyata don zubar da ƙura a wasu yanayi.

Organizationungiyar forasa don Rare Rashin Lafiya (NORD) - rarediseases.org

Mutuwa galibi tana faruwa ne a cikin shekaru 10 na farko na rayuwa, daga cututtukan da ake dadewa (masu ɗorewa) ko kuma saurin cutar da ke haifar da kamuwa da cutar lymphoma. Koyaya, wasu yaran da abin ya shafa sun rayu tsawon lokaci.

Matsaloli na iya haɗawa da:

• Yawaitar cututtuka da ke haɗuwa da wasu nau'ikan ƙwayoyin cuta
• Cutar kamar ta Lymphoma kamar ta cututtukan ƙwayoyin cuta kamar EBV
• Mutuwar farko

Kira mai ba ku sabis idan kuna da tarihin iyali na wannan matsalar kuma kuna shirin haihuwar yara.

Yi magana da mai ba ka sabis idan ɗanka ya nuna alamun cutar Chediak-Higashi.

Ana ba da shawarar ba da shawara kan kwayar halitta kafin yin ciki idan kuna da tarihin iyali na Chediak-Higashi.

Coates TD. Rashin lafiya na aikin phagocyte. A cikin: Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Nelson Littafin koyar da ilimin yara. 21st ed. Philadelphia, PA: Elsevier; 2020: babi na 156.

Dinauer MC, Coates TD. Rashin lafiya na aikin phagocyte. A cikin: Hoffman R, Benz EJ, Silberstein LE, et al, eds. Hematology: Ka'idoji da Aiki. 7th ed. Philadelphia, PA: Elsevier; 2018: babi na 50.

Toro C, Nicoli ER, Malicdan MC, Adams DR, Introne WJ. Ciwon Chediak-Higashi. Ra'ayoyin Gene. 2015. PMID: 20301751 www.ncbi.nlm.nih.gov/pubmed/20301751. An sabunta Yuli 5, 2018. An shiga Yuli 30, 2019.